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MNGIE: Muscle Pathology

Histochemistry Ultrastructure

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Muscle fibers with Mitochondrial Proliferation
  Irregular red stain (Ragged red)

Gomori trichrome stain

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H&E stain

Muscle fibers   Sizes: Mildly varied; Some small fibers
  Internal architecture: Irregular in some muscle fibers (Arrow)

H&E stain

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Muscle fibers with Mitochondrial Proliferation
  Internal architecture: Irregular

H&E stain

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VvG stain

Muscle fibers with Mitochondrial Proliferation
  Internal architecture: Irregular

VvG stain

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Lipid Droplets
  Mildly/Moderately increased in muscle fibers

Sudan black stain

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COX stain

Cytochrome Oxidase
  Reduced staining in scattered muscle fibers

COX stain

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COX stain
  Muscle fibers with reduced staining
  Mitochondria not visible

COX stain

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Succcinate Dehydrogenase (SDH)
  Scattered fibers with increased staining

SDH stain

SDH stain

Succcinate Dehydrogenase (SDH)
  Muscle fibers have increased staining
  Mitochondria appear large

SDH stain

Succcinate Dehydrogenase (SDH)
  Scattered muscle fibers with increased staining
  Mitochondria appear large

SDH stain

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Succcinate Dehydrogenase (SDH)
  Perimysial vessels: Normal staining
  Differs from increased staining in MELAS

SDH stain

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MNGIE: Muscle fiber types
  Type I muscle fibers: Mild predominance
  Lipid droplets: Increased in a few type 1 (Intermediate-stained) muscle fibers

ATPase pH 9.4 stain

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PAS stain

MNGIE: Normal glycogen in muscle fibers

PAS stain

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MNGIE: Muscle Ultrastructure

From: R Schmidt

MNGIE
  Mitochondria: Moderate proliferation associated with lipid droplets

From: R Schmidt

From: R Schmidt

From: R Schmidt

MNGIE
  Mitochondria: Proliferation; Mildly large; Round shape

From: R Schmidt

From: R Schmidt

MNGIE
  Mitochondria: Proliferation; Mildly large; Round shape, Clustered

From: R Schmidt

From: R Schmidt

MNGIE
  Mitochondria: Marked proliferation; Contain paracrystalline arrays (Parking lot inclusions)

From: R Schmidt

From: R Schmidt

MNGIE
  Mitochondria: Marked proliferation; Contain paracrystalline arrays (Parking lot inclusions); Whorls

From: R Schmidt

MNGIE
  Mitochondria: Whorled internal architecture with parking lot inclusions

From: R Schmidt

From: R Schmidt

MNGIE
  Mitochondria: Contain paracrystalline arrays (Parking lot inclusions)

From: R Schmidt

From: R Schmidt

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Return to: MNGIE

5/26/2026